Scleroderma in Pigmented Skin Populations

  • Lee Shapiro
  • Lesley Ann Saketkoo
  • Virginia D. Steen


Scleroderma, or more accurately systemic sclerosis (SSc), is a multi-organ systemic disease involving inflammation, vascular destruction, and disrepair with collagen deposition and fibrosis. Early recognition leading to timely management positively impacts survival and disease progression.



The authors wish to gratefully acknowledge the editorial and research contributions of Roberta Lukasiewicz, Heather Sickler, and Tammy Kelly to the development and writing of this chapter.


  1. 1.
    Oyoo O, Moots RJ, Ganda B. Stepping into the state of rheumatology in East Africa. Rheumatology (Oxford). 2012;51:1345–6.CrossRefGoogle Scholar
  2. 2.
    Chifflot H, Fautrel B, Sordet C, et al. Incidence and prevalence of systemic sclerosis: a systematic literature review. J Semin Arthritis Rheum. 2008;37:223–35.CrossRefGoogle Scholar
  3. 3.
    Dia D, Dieng MT, Sy TN, et al. Systemic scleroderma: 92 cases in Dakar. Dakar Med. 2003;48:82–6.PubMedGoogle Scholar
  4. 4.
    Adelowo OO, Oguntona S. Scleroderma (systemic sclerosis) among Nigerians. Clin Rheumatol. 2009;28:1121–5.CrossRefPubMedGoogle Scholar
  5. 5.
    Mijiyawa M, Amanga K, Oniankitan OI, et al. Connective tissue diseases in the hospital outpatient service in Lomé (Togo). Rev Med Interne. 1999;20:13–7.CrossRefPubMedGoogle Scholar
  6. 6.
    Pitche P, Amanga Y, Koumouvi K, et al. Scleroderma in a hospital setting in Togo. Med Trop. 1998;58:65–8.Google Scholar
  7. 7.
    Tager RE, Tikly M. Clinical and laboratory manifestations of systemic sclerosis (scleroderma) in Black South Africans. Rheumatology (Oxford). 1999;38:397–400.CrossRefGoogle Scholar
  8. 8.
    Steen VD, Oddis CV, Conte CG, Janoski J, Casterline GZ, Medsger TA Jr. Incidence of systemic sclerosis in Allegheny County, Pennsylvania. A twenty-year study of hospital-diagnosed cases, 1963-1982. Arthritis Rheum. 1997;40:441–5. PMID: 90829319.CrossRefPubMedGoogle Scholar
  9. 9.
    Steen V, Domsic RT, Lucas M, Fertig N, Medsger TA Jr. A clinical and serologic comparison of African American and Caucasian patients with systemic sclerosis. Arthritis Rheum. 2012;64:2986–94. PMID: 225766201.CrossRefPubMedPubMedCentralGoogle Scholar
  10. 10.
    Reveille JD, Durban E, Goldstein R, Moreda R, Arnett FC. Racial differences in the frequencies of scleroderma-related autoantibodies. Arthritis Rheum. 1992;35:216–8. PMID: 1734910.CrossRefPubMedGoogle Scholar
  11. 11.
    Laing TJ, Gillespie BW, Toth MB, et al. Racial differences in scleroderma among women in Michigan. Arthritis Rheum. 1997;40:734–42.CrossRefPubMedGoogle Scholar
  12. 12.
    Beall AD, Nietert PJ, Taylor MH, et al. Ethnic disparities among patients with pulmonary hypertension associated with systemic sclerosis. J Rheumatol. 2007;34:1277–82.PubMedGoogle Scholar
  13. 13.
    Mayes MD. Scleroderma epidemiology. Rheum Dis Clin N Am. 1996;22:751–64.CrossRefGoogle Scholar
  14. 14.
    Mayes MD, Lacey JV Jr, Beebe-Dimmer J, et al. Prevalence, incidence, survival, and disease characteristics of systemic sclerosis in a large US population. Arthritis Rheum. 2003;48:2246–55.CrossRefPubMedGoogle Scholar
  15. 15.
    Medsger TA Jr, Masi AT. The epidemiology of systemic sclerosis (scleroderma) among male U.S. veterans. J Chronic Dis. 1978;31:73–85.CrossRefPubMedGoogle Scholar
  16. 16.
    Wang J, Assassi S, Guo G, et al. Clinical and serological features of systemic sclerosis in a Chinese cohort. Clin Rheumatol. 2013;32:617–21.CrossRefPubMedGoogle Scholar
  17. 17.
    McNeilage LJ, Youngchaiyud U, Whittingham S. Racial differences in antinuclear antibody patterns and clinical manifestations of scleroderma. Arthritis Rheum. 1989;32:54–60.CrossRefPubMedGoogle Scholar
  18. 18.
    Pakunpanya K, Verasertniyom O, Vanichapuntu M, et al. Incidence and clinical correlation of anticentromere antibody in Thai patients. S Clin Rheumatol. 2006;25:325–8.CrossRefGoogle Scholar
  19. 19.
    Krishnamurthy V, Porkodi R, Ramakrishnan S, et al. Progressive systemic sclerosis in south India. J Assoc Physicians India. 1991;39:254–7.PubMedGoogle Scholar
  20. 20.
    Kumar A, Malaviya AN, Tiwari SC, et al. Clinical and laboratory profile of systemic sclerosis in northern India. J Assoc Physicians India. 1990;38:765–8.PubMedGoogle Scholar
  21. 21.
    Pradhan V, Rajadhyaksha A, Nadkar M, et al. Clinical and autoimmune profile of scleroderma patients from Western India. Int J Rheumatol. 2014;2014:983781.CrossRefPubMedPubMedCentralGoogle Scholar
  22. 22.
    Sharma VK, Trilokraj T, Khaitan B, et al. Profile of systemic sclerosis in a tertiary care center in North India. Indian J Dermatol Venereol Leprol. 2006;72:416–20.CrossRefPubMedGoogle Scholar
  23. 23.
    Eason RJ, Tan PL, Gow PJ. Progressive systemic sclerosis in Auckland: a ten year review with emphasis on prognostic features. Aust NZ J Med. 1981;11:657–62.CrossRefGoogle Scholar
  24. 24.
    Le Guern V, Mahr A, Mouthon L, et al. Prevalence of systemic sclerosis in a French multi-ethnic county. Rheumatology (Oxford). 2004;43:1129–37.CrossRefGoogle Scholar
  25. 25.
    Kuwana M, Okano Y, Kaburaki J, et al. Racial differences in the distribution of systemic sclerosis-related serum antinuclear antibodies. Arthritis Rheum. 1994;37:902–6.CrossRefPubMedGoogle Scholar
  26. 26.
    Nietert PJ, Silver RM, Mitchell HC, et al. Demographic and clinical factors associated with in-hospital death among patients with systemic sclerosis. J Rheumatol. 2005;32:1888–92.PubMedGoogle Scholar
  27. 27.
    Rocha LF, Luppino Assad AP, Marangoni RG, et al. Systemic sclerosis and silica exposure: a rare association in a large Brazilian cohort. Rheumatol Int. 2016;36:697–702.CrossRefPubMedGoogle Scholar
  28. 28.
    Bernatsky S, Smargiassi A, Johnson M, et al. Fine particulate air pollution, nitrogen dioxide, and systemic autoimmune rheumatic disease in Calgary, Alberta. Environ Res. 2015;140:474–8.CrossRefPubMedPubMedCentralGoogle Scholar
  29. 29.
    Jimenez SA, Diaz A, Khalili K. Retroviruses and the pathogenesis of systemic sclerosis. Int Rev Immunol. 1995;12:159–75.CrossRefPubMedGoogle Scholar
  30. 30.
    Neidhart M, Kuchen S, Distler O, et al. Increased serum levels of antibodies against human cytomegalovirus and prevalence of autoantibodies in systemic sclerosis. Arthritis Rheum. 1999;42:389–92.CrossRefPubMedGoogle Scholar
  31. 31.
    Marie I, Gehanno JF. Environmental risk factors of systemic sclerosis. Semin Immunopathol. 2015;37:463–73.CrossRefPubMedGoogle Scholar
  32. 32.
    Maître A, Hours M, Bonneterre V, et al. Systemic sclerosis and occupational risk factors: role of solvents and cleaning products. J Rheumatol. 2004;31:2395–401.PubMedGoogle Scholar
  33. 33.
    Cowie RL, Dansey RD. Features of systemic sclerosis (scleroderma) in South African goldminers. S Afr Med J. 1990;77:400–2.PubMedGoogle Scholar
  34. 34.
    Del Papa N, Quirici N, Scavullo C, et al. Antiendothelial cell antibodies induce apoptosis of bone marrow endothelial progenitors in systemic sclerosis. J Rheumatol. 2010;37:2053–63.CrossRefPubMedGoogle Scholar
  35. 35.
    Riemekasten G, Philippe A, Näther M, et al. Involvement of functional autoantibodies against vascular receptors in systemic sclerosis. Ann Rheum Dis. 2011;70:530–6.CrossRefPubMedGoogle Scholar
  36. 36.
    Baroni SS, Santillo M, Bevilacqua F, et al. Stimulatory autoantibodies to the PDGF receptor in systemic sclerosis. N Engl J Med. 2006;354:2667–76.CrossRefPubMedGoogle Scholar
  37. 37.
    Riemekasten G, Cabral-Marques O. Antibodies against angiotensin II type 1 receptor (AT1R) and endothelin receptor type A (ETAR) in systemic sclerosis (SSc)-response. Autoimmun Rev. 2016;15:935.CrossRefPubMedGoogle Scholar
  38. 38.
    Distler JH, Gay S, Distler O. Angiogenesis and vasculogenesis in systemic sclerosis. Rheumatology (Oxford). 2006;45(Suppl 3):iii26–7. Erratum in: Rheumatology (Oxford) 2008;47:234–35.Google Scholar
  39. 39.
    Tsou PS, Rabquer BJ, Ohara RA, et al. Scleroderma dermal microvascular endothelial cells exhibit defective response to pro-angiogenic chemokines. Rheumatology (Oxford). 2016;55:745–54.CrossRefGoogle Scholar
  40. 40.
    Bogatkevich GS, Ludwicka-Bradley A, Highland KB, et al. Impairment of the antifibrotic effect of hepatocyte growth factor in lung fibroblasts from African Americans: possible role in systemic sclerosis. Arthritis Rheum. 2007;56:2432–42.CrossRefPubMedGoogle Scholar
  41. 41.
    Bogatkevich GS, Highland KB, Akter T, et al. The PPARγ agonist rosiglitazone is antifibrotic for scleroderma lung fibroblasts: mechanisms of action and differential racial effects. Pulm Med. 2012;2012:545172.CrossRefPubMedGoogle Scholar
  42. 42.
    Silver RM, Bogatkevich G, Tourkina E, et al. Racial differences between blacks and whites with systemic sclerosis. Curr Opin Rheumatol. 2012;24:642–8.CrossRefPubMedPubMedCentralGoogle Scholar
  43. 43.
    Van den Hoogen F, Khanna D, Fransen J, et al. 2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative. Ann Rheum Dis. 2013;72:1747–55.CrossRefPubMedGoogle Scholar
  44. 44.
    Maricq HR, Weinrich MC. Diagnosis of Raynaud’s phenomenon assisted by color charts. J Rheumatol. 1988;15:454–9.PubMedGoogle Scholar
  45. 45.
    Gelber AC, Manno RL, Shah AA, et al. Race and association with disease manifestations and mortality in scleroderma: a 20-year experience at the Johns Hopkins Scleroderma Center and review of the literature. Medicine (Baltimore). 2013;92:191–205.CrossRefGoogle Scholar
  46. 46.
    Nusbaum JS, Gordon JK, Steen VD. African American race associated with body image dissatisfaction among patients with systemic sclerosis. Clin Exp Rheumatol. 2016;34 Suppl 100:70–3.PubMedGoogle Scholar
  47. 47.
    Cannick L, Douglas G, Crater S, et al. Nodular scleroderma: case report and literature review. J Rheumatol. 2003;30:2500–2.PubMedGoogle Scholar
  48. 48.
    Ramos PS, Silver RM, Feghali-Bostwick CA. Genetics of systemic sclerosis: recent advances. Curr Opin Rheumatol. 2015;27:521–9.CrossRefPubMedPubMedCentralGoogle Scholar
  49. 49.
    Black HR, Quallich H, Gareleck CB. Racial differences in serum creatine kinase levels. Am J Med. 1986;81:479–87.CrossRefPubMedGoogle Scholar
  50. 50.
    Spechler SJ, Jain SK, Tendler DA, et al. Racial differences in the frequency of symptoms and complications of gastro-oesophageal reflux disease. Aliment Pharmacol Ther. 2002;16:1795–80.CrossRefPubMedGoogle Scholar
  51. 51.
    Segal I. The gastro-oesophageal reflux disease complex in sub-Saharan Africa. Eur J Cancer Prev. 2001;10:209–12.CrossRefPubMedGoogle Scholar
  52. 52.
    Sonnenberg A. Effects of environment and lifestyle on gastroesophageal reflux disease. Dig Dis. 2011;29:229–34.CrossRefPubMedGoogle Scholar
  53. 53.
    Nietert PJ, Mitchell HC, Bolster MB, et al. Racial variation in clinical and immunological manifestations of systemic sclerosis. J Rheumatol. 2006;33:263–8.PubMedGoogle Scholar
  54. 54.
    McNearney TA, Reveille JD, Fischbach M, et al. Pulmonary involvement in systemic sclerosis: associations with genetic, serologic, sociodemographic, and behavioral factors. Arthritis Rheum. 2007;57:318–26.CrossRefPubMedGoogle Scholar
  55. 55.
    Greidinger EL, Flaherty KT, White B, et al. African-American race and antibodies to topoisomerase I are associated with increased severity of scleroderma lung disease. Chest. 1998 Sep;114:801–7.CrossRefPubMedGoogle Scholar
  56. 56.
    Aggarwal R, Lucas M, Fertig N, et al. Anti-U3 RNP autoantibodies in systemic sclerosis. Arthritis Rheum. 2009;60:1112–8.CrossRefPubMedGoogle Scholar
  57. 57.
    Shapiro LS, Saketkoo L, Farrell JF, et al. Development of a “Renal Crisis Prevention Card” as an educational tool aimed at improving outcomes in high-risk patients with systemic sclerosis. Abstract 2716 ACR/ARHP Annual Meeting, November 2014; Boston, MA.Google Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Lee Shapiro
    • 1
  • Lesley Ann Saketkoo
    • 2
  • Virginia D. Steen
    • 3
  1. 1.Division of RheumatologyAlbany Medical CollegeAlbanyUSA
  2. 2.Clinical Immunology, Allergy and Rheumatology, Pulmonary Diseases, Critical Care and Environmental MedicineNew Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, Tulane University School of Medicine, Tulane Lung CenterNew OrleansUSA
  3. 3.Department of MedicineGeorgetown University Medical Center, Pasquerilla Healthcare CenterWashington, DCUSA

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